Daddy I’m ready…………..

At night I lay in bed with this little girl, holding her hand, simply amazed at how much I love her. How could you ever know before becoming a parent how your heart could grow, and grow ….and grow every day, consumed with so much emotion. As Sophia’s dad I have learned so much about life from this precious little package. There is not a single thing in this world that I would not do for my family. Today I have learned that sometimes you have to nudge your heart out of the way in order to make the best decision for your child. In the beginning of our journey with Spinal Muscular Atrophy, I was the parent who was prepared for dealing with the diagnosis and learning the best care options for Sophia. We were both extremely devastated and Catherine just needed a little more time than I did to grasp the realities of the disease. This time however, I was unprepared to face the fact that Sophia needed to be trached. I am so incredibly thankful to my beautiful wife for allowing me the time to come to terms with what needs to be done, and for allowing me to come to a place of acceptance.

Last year in May,we started noticing that Sophia was beginning to struggle to come off of her Bipap. She started to labor off of Bipap and would start to become extremely sweaty on her eyebrow and forehead. What seemed like over night, Sophia became dependent. We could no longer take Sophia off without her oxygen saturation levels plummeting and her heart rate would quickly follow. We tried to slowly wean Sophia off to no avail. In June I had called Dr Schroth and told her what we were seeing. She was surprised and upset since Sophia had looked so good at her visit in May. Over the next few months we began speaking to many other SMA families offering advice on different ways to wean Sophia. Nothing would work. We had multiple chest x-rays and Sophia has always been clear, I was hoping to see something that would point to a direct cause of Sophia’s dependency. In September Dr Schroth was in town and was nice enough to come by to see Sophia. While she was here I wanted her to see what happened when we took the Bipap off of Sophia, after witnessing Sophia immediately drop Dr Schroth informed us that for Sophia the safest option was for her to be trached. Catherine and I cried and Dr. Schroth consoled us telling us we had done all we could but unfortunately it was a part of the disease. After seeing Dr Schroth we wanted to get a second opinion on Sophia. Later that month we took Sophia to see Dr Bach and Brian Weaver. At that visit we tested Sophia’s vital lung capacity……..it was a .5 and Dr Bach informed us that unfortunately Sophia would probably never return to being able to come off of Bipap. Brian Weaver had suggested a Trache for Sophia’s safety and Dr. Bach informed us Sophia could possibly maintain just being on 24/7 Bipap. These were some of the most highly respected professionals in our disease, but I still wanted to believe in my heart that there could be a chance Sophia would somehow bounce back.

Living the past year with Sophia’s condition has been extremely challenging both emotionally and physically. Sophia’s dependency was every second of every day. To bathe Sophia and to wash her hair required both of us. I would carry Sophia to the bathroom, trying to support her in a flat position while Catherine would tailgate behind carrying the Trilogy. When I would gently place her on her bath chair Catherine would set the machine down and begin to wash Sophia’s body. When we were ready for Sophia’s face and hair we would remove Sophia’s headgear. I would hold the bubble mask on her nose while Catherine washed her hair. It was tricky to keep her nose in the mask while her head was moving from being shampooed. When bath time was done we would tell Sophia to take 3 breaths and to hold it…………..I would pick Sophia up and Catherine would follow. As soon as we would get her to the bedroom we would put the mask on Sophia’s face. I would hold the mask on Sophia while Catherine would blow dry her hair. Once we were done we were able to put a new dry headset back on……..and you could see the relief in Sophia’s face. The truth is for the last year we could not take the mask off for a minute, not for a family picture, and not for a kiss. It has been a long year since I have been able to rub my cheek against my daughter’s. A year since I have been able to rub my nose against hers. A year since I have been able to run my fingers through her hair without the headgear in the way. The fear that consumed us was Sophia’s health. When SMA kids are sick……they are generally on Bipap 24/7 till they are better. What scared me was ………………..what happens to an SMA child who is already on Bipap 24/7 who gets sick? What started to make me see that Sophia needed a secure airway was the look of absolute panic on her face every time her mask was removed.

Sophia had recently started to have really bad bloody noses. Every time we would cough her the cough mask would turn red. The suction lines and canister would turn bright red. Sophia would begin to choke on her blood and you are stuck as a parent. You know you need to keep coughing her to clear her, but the more you coughed the more she would bleed. Her dependency over the past year 24/7 was not allowing her nose to heal. It has now become every set that we have scary bleeding episodes. Sophia knew she would bleed and choke. Now every time the Cough machine would go on Sophia would start to cry with tears streaming down her face. On Saturday I knew in my heart that it was time. Sophia was suffering. I looked at the quality of her life, having a mask that could not come off her face, always in her line of view. The mask was always there. I would not want to live like that, the constant fear……….so why should she.

I know we are making the right choice for our daughter, but it comes with so much fear and trepidation. I love the cute sounds Sophia makes when she is calling me and I fear never hearing her voice again. I fear that Sophia may not like the trache, even though I know she has to like it better than the mask at this point. I fear that because Sophia has a tube in her throat rather than a mask on her face that my daughter will be disqualified from future potential trials. I know that I must make the right decisions for Sophia today in the here and now, and I know this is the right choice. I know that I am not ready to lose her, and she still wants to be here. On Thursday we will be taking Sophia to the hospital to be admitted and Sophia will be trached on Friday.

I know we are doing the right thing for our child……………and yet it still weighs so heavy on my heart.

Thank you to this wonderful community for all of your support. Thank you Sarah Turnbull and Jana Gundy for making and sending us videos to ease us through this process.

This was the last picture we had of Sophia without her mask……cannot wait to see this face again!!!

Comments

God bless Vinny. You are all in my thoughts and prayers at this incredibly difficult time. I am sure you are doing the right thing, for all of you and for Sophia. It will be good to see her beautiful face unobstructed again. (((((hugs)))))

Oh you guys….I am so sorry. All we can do for our children is our best and Sophia feels that from you EVERY day. She is a lucky little girl to have so much love for her. Although this is very scary time and your hearts are hurting, know that all of your SMA family is here for you! I constantly draw inspiration and strength from you in our own journey. I can’t wait to see the first picture of Sophia with her mask off!

Owen has been BiPAP dependent since he was about four years old. (He’s now 8 1/2.) If he comes off of the Trilogy or LTV for more than, say, twenty or thirty seconds, he’ll desat and start to arrest. But he’s weathered many a respiratory virus on BiPAP, using the nasal aires rather than a mask, which he never tolerated.

There are plusses and minuses to trachs — so far I’ve been happy to avoid one. I suspect Dr. Back is correct that she could handle it. Just a thought.

Vinny, just know that the choices you make for Sophia are in her best interest. You are doing what is best for your precious girl and I know she will love and thank you for this when it is all said and done. Jake and I will be saying prayers on Friday. You and Catherine are fabulous parents, never doubt your decisions. Jennifer

I am so very sorry. Sophia is a lovey little girl and she is blessed to have such brave, caring parents. I know little about SMA. I do, however, know about trachs. I was trached at age 60 in order to save my life. Learning to live with this tube protruding from my throat was not easy but is certainly doable. After awhile I began to not even know it was there. Sophia will feel so much better with her trach. Is it a pleasant choice? No it is not. However the trach will provide needed oxygen to her precious body. Always consider the alternative and be thankful for the technology that allows you to keep your precious daughter. God bless you all. Rely on Him and He will see you through this awful time.

I know this was a difficult journey and decision to make. Please know that I am here for you in any way I can be. Transitioning to a trach was overall soooooo easy for us. Difficult emotionally at first, but seeing how happy, safe and healthy Ally has been has been so rewarding. Having NO more masks is amazing. Please do not hesitate to call or email if i can help in any way. (((( Hugs to all of you)))

My precious Savanna has been trached since right before her 1st birthday. She just could not tolerate the BiPap because it was giving her such terrible skin breakdown and she was only on the Bipap for a few weeks. I hated the Bipap. It covered her face and her mouth and she hated it. What I found was that Savanna was happier when she was intubated because it freed up her face. We too had a difficult decision to make on whether or not to trach her. That has been almost 9 years ago and I thank God daily that we made that decision. Savanna has a wonderful life. She drives her power wheelchair, with the vent attached to the back. Her beauitful face is free from any masks and her curly hair falls around her face with everyone telling her how beautiful her hair is. She is able to talk with her trach. And boy, does she talk….nonstop at home and singing too. It was an adjustment for us but we had never had Bipap or anything at home. She became ill and went downhill so fast, we didn’t have many options. Once she started to recover from the illness, her main problem was just that she could not handle her secretions. She was literally drowning on her secretions and a nurse practitioner suggested a trach to make it easier to keep her airway clear. I freaked out at the prospect but soon realized that it was not so bad after all. Savanna goes in the swimming pool, goes to public school (all day), goes to movies, shows, concerts, the zoo, the lake, just about everything any other kid does. I think the only thing I haven’t been able to do is take her to a water park! I will keep your beautiful Sophia and your family in my prayers and will tell Savanna so that she can pray for her as well. Just know that as a parent, you always make the right decision. No one knows your child better than you and your wife and you know what is best for her. Just know that this trach family is here if you need anything at all! Blessings!

Vin, you and catherine are wonderful parents. I know the fear but also know the joy of not so much worry on an everyday basis. I had tears reading your post and looking at that beautiful picture of Sophia! I can’t wait to see the pics of the little princess without her mask. I have great faith that she will do wonderfully.
We will keep you all in our prayers.

You are making the right choice, there is no doubt about that. Llarell was trached since he was 10 months old. we never liked the mask, and he just used it for two consecutive days. i remember the face he had before, and after the surgery. before the surgery, he winked his eyes, you know, like when they say that “everything will be fine”, kind of a wink…after some hours, he came out from surgery, and, still under the effects of the anesthesia, he opened his eyes right after my voice, and he smiled. then he fell asleep. the next day, we had the biggest smile we had seen in months. up to this point, he is a happy and healthy kid, and never went back to the hospital.
there are many fears you have, most of them for cultural ambivalences and misconceptions about tracheostormy care. can’t blame you, I was right there where you are. I will be more than happy to help you out with this, if you lt me. Summer is coming, and I won’t hesitate to go to New York to be with you. but just as first thing, she will make noises, and as she grows older, she might say some words. you will keep on hearing sophia. vocal cords are above the trache, and they won’t be touched during surgery.
you will see the quality of life you will offer. Llarell has been on the beach, pools, roller coasters, camping, 4 airplanes, 1 cruise ship, unlimited amount of mall visits, christmas parties all over the place… he loves to party. trache will give you this freedom…then again, I will be more than happy to help you out.
at this point, don’t play doctor, or nurse…just be DAD. God bless you!

Thanks for sharing your heart, Vinny! You guys will be pros at it and saying how easy it is in a few months! I can’t wait to see Sophia’s beautiful face again . . . I remember how Stella’s face filled out so beautifully after having had the bipap on it. As for her voice, I bet she will still be calling you! You should have heard Stella tonight during Dancing with the Stars! She was a chatterbox and even louder than she was before her trach. Hugs and love! Don’t hesitate to call!

Sending huge prayers for your family and your precious girl for your new part of the journey. I know you will handle it with poise and grace. Sophia is so blessed to have parents that work so hard to make the most of each day.

Greetings,
Thanks for sharing your heart so openly. As an adult with SMA, it truly touches my heart to hear how deeply you value your daughter’s every little ability and possibility! Having said that, you definitely made the right decision for Sophia. Although I don’t use a vent or trach, I do know first hand that those of us with SMA are always doing a balancing act as we try to keep strength and stay healthy. With the trach, she won’t have to worry and she’ll have so much more energy to use on living life rather than on just staying alive!
Blessings to all of you, Ellyn

My eyes are overflowing with tears at what you and Catherine are feeling. No one knows a child better than their own parent and I’m sure you and Catherine are making the best decisions for Sophia. You have met with the best in the SMA field and it seems they would not lead you wrong. Our hearts, love and support are with Sophia, you and Catherine at this stressing time. Hugs and love. Jen, Chris and Hayden

Catherine and Vinny, I can not imagine what you are going threw but as an SMA parent, or any parent we want to do whats best for our children! The only thing I can say is: You have to do a lot of soul searching and I think you both have done that. It is very hard to walk the unknown but even harder when it is someone care for very deeply for. Sophie is a very special and lucky princes to have angels here on earth to hold her hand threw this journey. The Gaynor’s are fighters:) My thoughts and prayers are with you always but will be in high gear from now until you post after surgery. If you need anything in the Wisconsin area please don’t be afraid to ask.

What an inspiring story…one identical to our’s..My precious Grandson Conor at 3 years of age was traeched…After being on Bi-Pap since diagnosis at 4months old…He was virtually 24/7 on bi-pap,which he didn’t like as he got older as it got in his way of doing normal things…watching tv,playing with his toys etc..His Mummy & Daddy had the same dilemma and knew in order to give Conor a chance at the life he loves..a traeche was indeed the Only option…they didn’t think of the only other option!Well suffice to say 5 YEARS on our brave boy is sure living life to the full!He goes to full-time school in his powerchair with his Nippy Vent on the back of it…Loves visiting the Cinema,Zoo,Bowling and doing everything an 8 year old likes to do!Your decision is so difficult,one that no parent should have to do,but this cruel illness has to be lived with so therefore give these special heaven-sent kids the right to breathe(albeit aided)and to spend their days on earth with the famillies who adore them…Please let there be a CURE sooner rather than LATER for all of the SMA Community.As i have always said,until a famous person in the public eye ie:Actor,Sportsman,Politician,Singer,has a child with SMA it will remain an illness only known to the people who live with it and their family and friends…Godbless your Family..Julie Durkin,Conor’s proud Nan..Liverpool,England..

My heart hurts for you and your family. This is a decision no parents should have to make for their children. Your post puts everything into perspective and allows me to see how minor all of life’s others issues really are. Sending strength and healing thoughts to Sophia during this transition.

Like so many, we too will be thinking about you guys as you enter the next stage w/ Sophia. I’m sure it’s not been an easy choice, but you are doing what you feel is best for your daughter and many people support you. Praying for a smooth procedure, and a quick recovery/healing process. Sending strength and blessings.

You and Catherine are both doing the best you can for your beautiful daughter. These are hard choices but you are making the right ones, based on careful consideration. Hugs and prayers for all of you.

I know you’re making the right decision. We have an SMA Type 1 son. When he was 8, his lung collapsed. He happened to be in the hospital with pneumonia at the time. Winters were the worst, It seemed like he was in the hospital for months, but actually he was usually in for at least 21 days at a time. We had no choice one night when his lung collapsed. The doctor said “We can make him comfortable and we can let him go, let nature take it’s course, or we can trach him and put him on a vent. He’ll probably have about a 10% chance of survival if we vent him.” I thought for a few minutes and said to myself 10% is better than 0% chance…so trach and vent him.” You know, he perked right up within hours. He has only been hospitalized once with pneumonia since then…and just as importantly, he’s VERY healthy and happy. My husband and I were terrified of the vent and of a trach. We were always told that the vent was the last resort, but it came down to the last resort. But, please remember this…our son’s quality of life is infinitely better than it was before the vent and trach! He is so much healthier. He was using all of his energy just to breathe and survive. He was sick all the time and he was very thin and just fragile. He began gaining weight after, he finally had energy to do other things besides just survive. We actually were able to hear him cry and laugh, something he never had enough “air” to do before! I am very happy to report to you that our son,….our little SMA Type 1 baby turned 24 in April…thank GOD for that trach and the vent..and the wonderful medical staff at that hospital! Don’t you worry, I can almost guarantee that Sophia will thrive and be so much healthier (and happier) with the trach. She will be able to have you love on her, and she won’t have to have that mask on 24/7. I can’t even imagine how that feels. You must be so thrilled at the prospect of holding her, kissing her, running your fingers through her hair…and no panic when the mask is off.

Our son is a wonderful, happy, healthy 24 year old. He has an electric wheelchair that he uses, he is an avid gamer and he loves life! He’s teaching himself Japanese and has many interests. We are very blessed indeed!

On another note, our son’s airway is crooked and we had a lot of trouble changing the trach, but my husband came up with a system that works wonderfully. If you are interested, please email me and I will explain it to you. As a matter of fact, tomorrow is trach change day, I think I will try to film it. It’s very simple and it’s so much easier on nerves, not having to worry if the trach is going to go in “this time.” Good luck, please keep us all informed how things go. I think you may be in for some surprisingly amazing healthy times!
Sincerely,
Debbie Cattivera

You guys are a guiding light for us. Your love and strength holds all of us up when we need it. Let us hold you up now. Do not doubt yourselves…..you & Catherine are amazing parents. You are showing the rest of us the “right”‘way to face this disease head on. I love you guys.

Thank you for sharing your story. You inspire so many with your courage, thoughtfulness, and warmth. Sophia couldn’t be in better hands and she couldn’t have better advocates. The decision and the procedure must weigh heavily on you as your family takes the next step in your journey. Know that she loves you as much as you love her, and her trust in you is well placed. You are in our thoughts and we send you all kinds hugs. Miss Getty sends Sophia smoochies!

Sharing your journey helps us all : those just beginning, those who are there and just as importantly… those of us who never knew. I am astonished at your strength and conviction. Your family holds a special place in my heart.

Prayers are with you during this time of transition. You will be a pro before you know it and see that the trach will open up a new world that i”m sure will be easier for you and Sophia more importantly!

I don’t know you, but I have been following your family’s and many other SMA families stories since November when I learned of this horrific nightmare of a disease from my dear friend whose infant son, Coby, was diagnosed with Type 1. I just wantd to say that I am in awe of your strength, your courage and your unrelenting love for your Sophia. There is no bigger love than that of a parent and child and a no bigger feeling of helplessness when we can’t make them better. But what you are doing is such a wonderful step in the quality of life for all of you that I can’t imagine a better choice. There are people praying for your beautiful daughter in all the little nooks and crannies of this world that you wouldn’t even imagine- and I am one of them. Thank you for sharing your story and your beautiful baby girl with us. Sophia, along with Coby Kulis and all the other warriors and angels, are making a huge impact on this disease. You should be SO proud. Take care and God Bless, Marisa Conte

dear catherine and vincent~
my prayers and loving thoughts are always with you both and sophia~
it is so encouraging to read of others that have had positive results~
with the trach.
sophia is, as others have said~loved by so many~*
we cry and continue to pray for both of you to endure and be a constant
ray of light in your dear one’s eyes…
….“may the God who gives hope fill you with all joy and peace by your believing,
that you may abound in hope with power of holy spirit.”—Rom. 15:13.